PEX19/YDL065C Summary Help

Standard Name PEX19 1
Systematic Name YDL065C
Alias PAS12
Feature Type ORF, Verified
Description Chaperone and import receptor for newly-synthesized class I PMPs; binds peroxisomal membrane proteins (PMPs) in the cytoplasm and delivers them to the peroxisome for subsequent insertion into the peroxisomal membrane; interacts with Myo2p and contributes to peroxisome partitioning (1, 2, 3, 4 and see Summary Paragraph)
Name Description PEroXisome related
Chromosomal Location
ChrIV:337277 to 336249 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Gene Ontology Annotations All PEX19 GO evidence and references
  View Computational GO annotations for PEX19
Molecular Function
Manually curated
Biological Process
Manually curated
Cellular Component
Manually curated
Regulators 3 genes
Classical genetics
Large-scale survey
157 total interaction(s) for 119 unique genes/features.
Physical Interactions
  • Affinity Capture-MS: 8
  • Affinity Capture-RNA: 2
  • Affinity Capture-Western: 3
  • Co-localization: 1
  • PCA: 20
  • Protein-peptide: 7
  • Reconstituted Complex: 5
  • Two-hybrid: 23

Genetic Interactions
  • Dosage Growth Defect: 1
  • Negative Genetic: 61
  • Positive Genetic: 18
  • Synthetic Growth Defect: 6
  • Synthetic Lethality: 2

Expression Summary
Length (a.a.) 342
Molecular Weight (Da) 38,706
Isoelectric Point (pI) 4.08
Phosphorylation PhosphoGRID | PhosphoPep Database
sequence information
ChrIV:337277 to 336249 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Last Update Coordinates: 2006-04-12 | Sequence: 2006-04-12
Subfeature details
Most Recent Updates
Coordinates Sequence
CDS 1..1029 337277..336249 2006-04-12 2006-04-12
Retrieve sequences
Analyze Sequence
S288C only
S288C vs. other species
S288C vs. other strains
External Links All Associated Seq | Entrez Gene | Entrez RefSeq Protein | MIPS | Search all NCBI (Entrez) | UniProtKB
Primary SGDIDS000002223

The biogenesis of peroxisomes requires a group of protein factors referred to as peroxins which are encoded by the PEX genes. Peroxisomal proteins are synthesized on free polyribosomes and imported posttranslationally. The transport of peroxisomal matrix proteins from the cytoplasm to the peroxisome is mediated by two peroxisome-targeting signal sequences (PTS1 and PTS2). Peroxisomal membrane proteins (PMPs) are imported independently of the matrix proteins by a distinct mechanism mediated by the membrane PTS signal (mPTS) (2, 5, 6, 7 and references therein).

In yeast, Pex19p and Pex3p are two proteins required for the proper localization and stability of PMPs (2). Pex19p is a farnesylated, hydrophilic and acidic protein that has chaperone and receptor functions (1, 7). It binds and stabilizes newly synthesized class I peroxisomal membrane proteins (PMPs) in the cytoplasm and delivers them to the peroxisome for subsequent insertion into the peroxisome membrane (8, 7 and references there in). Pex19p interacts with Pex3p in the ER during the early stages of de novo peroxisome biogenesis and is required for the exit of Pex3p from the ER (9, 10, 11). pex19 null mutants completely lack detectable peroxisomal membrane structures and mislocalize the PMPs to the cytosol where they are rapidly degraded (2).

Pex19p is evolutionarily conserved, and shares sequence similarity with human Pex19p (also known as PXF). The human peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous diseases characterized by severe mental retardation, neuronal, hepatic and renal abnormalities, and death in early infancy (12). Mutations in human PEX19 have been associated with Zellweger Syndrome and Refsum Disease.

Last updated: 2007-07-05 Contact SGD

References cited on this page View Complete Literature Guide for PEX19
1) Gotte K, et al.  (1998) Pex19p, a farnesylated protein essential for peroxisome biogenesis. Mol Cell Biol 18(1):616-28
2) Hettema EH, et al.  (2000) Saccharomyces cerevisiae pex3p and pex19p are required for proper localization and stability of peroxisomal membrane proteins. EMBO J 19(2):223-33
3) Fang Y, et al.  (2004) PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins. J Cell Biol 164(6):863-75
4) Otzen M, et al.  (2012) Pex19p contributes to peroxisome inheritance in the association of peroxisomes to Myo2p. Traffic 13(7):947-59
5) Sacksteder KA and Gould SJ  (2000) The genetics of peroxisome biogenesis. Annu Rev Genet 34:623-652
6) Purdue PE and Lazarow PB  (2001) Peroxisome biogenesis. Annu Rev Cell Dev Biol 17:701-52
7) Fujiki Y, et al.  (2006) Import of peroxisomal membrane proteins: The interplay of Pex3p- and Pex19p-mediated interactions. Biochim Biophys Acta 1763(12):1639-46
8) Girzalsky W, et al.  (2006) Pex19p-dependent targeting of Pex17p, a peripheral component of the peroxisomal protein import machinery. J Biol Chem 281(28):19417-25
9) Kragt A, et al.  (2005) Endoplasmic reticulum-directed Pex3p routes to peroxisomes and restores peroxisome formation in a Saccharomyces cerevisiae pex3Delta strain. J Biol Chem 280(40):34350-7
10) Hoepfner D, et al.  (2005) Contribution of the endoplasmic reticulum to peroxisome formation. Cell 122(1):85-95
11) Tam YY, et al.  (2005) Pex3p initiates the formation of a preperoxisomal compartment from a subdomain of the endoplasmic reticulum in Saccharomyces cerevisiae. J Biol Chem 280(41):34933-9
12) Matsuzono Y, et al.  (1999) Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly. Proc Natl Acad Sci U S A 96(5):2116-21